Chondrosarcoma is a unique kind of tumor that originates from the cartilage-producing neoplastic mesenchymal cells and appears in both the appendicular and atlantoaxial skeleton. It is the second most prevalent neoplastic bone tumor, with an occurrence of 0.79/100,000/year. The biological presentation of this cancer fluctuates extensively, depending on the grade and anatomical location. Since chondrosarcoma is predominantly resistant to conventional chemo- and radiation therapy, surgical resection remains the sole curative treatment, although at present new treatment modalities are under investigation.